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Long-term steroid treatment in two patients with acquired epileptic aphasia
of the children with cryptogenic spasms with hypsarrhythmia if we were to do a meticulous neurologic study.
76. OROLINGUAL D Y S K I N E S I A I N D U C E D BY CLONAZEPAM J. Campistol, E. Fernandez-Alvarez, and P, Poo, Barcelona. Spain In pediatric patients the administration of benzodiazepines frequently is associated with multiple and well-known adverse reactions. However, early orolingual dyskinesia had not been reported in relation with the usage of clonazepam. We present 6 patients, ages 2 months to 7 years, who showed extrapyramidal movements in the course of either monotherapy or polytherapy with clonazepam. The doses ranged from 0.1-0.9 mg/kg/day by oral or intravenous dose. A few days (2-30) after they were treated with clonazepam the patients presented with different types of abnormal movements of the lips almost continuously, imitating those of rabbits or especially a fish. Simultaneously intermittent protrusion of the tongue from the mouth and excessive salivation were observed. The lingual motility, as described above, impeded them from holding suckers in their mouths and caused difficulty in alimentation. Thus, a nasogastric tube was necessary for feeding 2 patients during this period. At the same time in the course of these abnormal movements, polygraphic EEG provided no evidence of paroxysmal alterations in any patient, Decreased doses of clonazepam or substitution by another benzodiazepine (clobazam) resulted in the disappearance of such episodes. We believe that such early dyskinesias are clearly the results of clonazepam and that they are not dosedependent, although they are more likely to occur when high doses are used. Apparently they do not leave sequelae (some patients are followed up to 5 years). It is essential to recognize these dyskinesias to avoid unnecessary investigations. They should not be confused with seizures in order to hinder more aggressive therapies. Instead, once we confirmed the diagnosis we recommended changing to another benzodiazepine (clobazam) or to reduce the doses of clonazepam.
77. LONG-TERM STEROID TREATMENT IN TWO PATIENTS WITH ACQUIRED EPILEPTIC APHASIA Roberto H. Caraballo, Eugenio Grillo, Ana M. Soprano, and Natalio Fejerman, Buenos Aires, Argentina Among a larger series of cases with acquired epileptic aphasia (AEA) we selected for treatment two patients on the basis of having been nonresponsive to different antiepileptic drugs and remaining aphasic for a long time. The age of onset of disease was 6 years in both cases and the delay between onset of AEA and steroid treatment was 15 and 18 months. At this time their sleeping EEGs still demonstrated continuous spike-and-wave activity covering over 85% of the slow sleep recording. Prednisone (1 mg/kg/day) was continued for 6 and 8 months. EEG normalized after 1 month of treatment in the first case. Discharges were less frequent after 2 months, with EEG becoming normal at 6 months in the second. Language response was seen much later: improved comprehension and isolated words appeared after 2 and 3 months, respectively. Almost normal language was recovered at 6 and 8 months of daily steroid treatment. In accordance with the results of other authors, we think that high-dose, prolonged steroid treatment should be attempted
364 PEDIATRIC NEUROLOGY Vol. 8 No. 5
in patients with AEA nonresponsive to antiepileplk drugs, iw cluding wdproic acid, cthosuximide, and benzodia/cpinc~. The first sign to look for is improvement of EEG and ~ c ,hould ,a air for it at least 2-3 months befl~re considering it to bc a m:atmenl failure. Spontaneous remission or changes in EEG abnormalities or in language are probably not operating in thesc 2 cases, considering the long period of persistent clinico-electroencephatographic features of AEA elapsed unlil steroid treatment was started.
78. CONGENITAL CEREBELLAR HYPOPLASIA WITH GRANULAR CELL ATROPHY I. Pascual-Castroviejo, M. Gutierrez, C. Morales Bastos, 1. Gonzalez Mediero, A. Martinez Bermejo, and J. Arcas. Madrid, Spain Eleven children (6 males, 5 females) are presented with cerebellar hypoplasia or atrophy and primary degeneration of the granular cell layer. This disease is a hereditary condition of a recessive autosomal nature and has definite clinical features: hypotonia, strabismus, very slow motor development, nonprogressive ataxia, retarded language development with dysarthria, and mental retardation. All neuroradiologic studies, pneumoencephalography, CT, and MRI, disclosed severe cerebellar hypoplasia. MRIs are the most revealing test and present great similarity among the cases. Necropsy study was performed in 3 of I I patients, and cerebellar hypoplasia with cell loss of the granular cell and diverse abnormalities of the Purkinje cells, was found in 2 patients. The third patient with a histologic study was a gM, sister of one of the 2 patients described above, who had a similar but shorter clinical course, dying at 3 months of age; the necropsy study demonstrated only localized atrophies of the cerebellar folia without microscopic changes in the granular cell layer. Her sister lived to age 5 years and presented with severe cerebellar hypoplasia with typical changes in the granular cell layer and Purkinje cells. The findings observed in our series and the study of cases described in the literature, suggest to us that there are two forms or types of this disease that differ mainly in that one form presents with microcephaly and lower mental level and the other evolves with a normal cephalic size and less severe mental retardation.
79. HYPOMELANOSIS OF ITO: MR][ RESULTS IN 14 PATIENTS A . L Martfnez Jim6nez, 1. Pascual-Castroviejo, E. Mufioz Hiraldo, A. Tendero, C. S~inchez Pina, and M.R. Garcfa Melian, Madrid, Spain During the last 25 years, 68 patients with hypomelanosis of Ito (HI) were studied in our service. MRI was performed in 14 patients (7 males, 7 females). MRI anomalies were found in 8 patients (57%). Intracerebral changes are located in any zone of the brain (i.e., cerebral hemispheres, basal ganglia, brainstem, and less frequently in the cerebellum). These alterations demonstrate several types of lesions, cortico-subcortical changes of the tuberous type (similar to those observed in tuberous sclerosis), hemimegalencephaly, changes of the type of cerebral heterotopies located in white matter of any zone of the cerebral hemispheres, periventricular white matter changes, retarded myelination, and cortico-subcortical atrophy of the temporal lobes. Although most lesions are observed on Tl-weighted images, the
76. OROLINGUAL D Y S K I N E S I A I N D U C E D BY CLONAZEPAM J. Campistol, E. Fernandez-Alvarez, and P, Poo, Barcelona. Spain In pediatric patients the administration of benzodiazepines frequently is associated with multiple and well-known adverse reactions. However, early orolingual dyskinesia had not been reported in relation with the usage of clonazepam. We present 6 patients, ages 2 months to 7 years, who showed extrapyramidal movements in the course of either monotherapy or polytherapy with clonazepam. The doses ranged from 0.1-0.9 mg/kg/day by oral or intravenous dose. A few days (2-30) after they were treated with clonazepam the patients presented with different types of abnormal movements of the lips almost continuously, imitating those of rabbits or especially a fish. Simultaneously intermittent protrusion of the tongue from the mouth and excessive salivation were observed. The lingual motility, as described above, impeded them from holding suckers in their mouths and caused difficulty in alimentation. Thus, a nasogastric tube was necessary for feeding 2 patients during this period. At the same time in the course of these abnormal movements, polygraphic EEG provided no evidence of paroxysmal alterations in any patient, Decreased doses of clonazepam or substitution by another benzodiazepine (clobazam) resulted in the disappearance of such episodes. We believe that such early dyskinesias are clearly the results of clonazepam and that they are not dosedependent, although they are more likely to occur when high doses are used. Apparently they do not leave sequelae (some patients are followed up to 5 years). It is essential to recognize these dyskinesias to avoid unnecessary investigations. They should not be confused with seizures in order to hinder more aggressive therapies. Instead, once we confirmed the diagnosis we recommended changing to another benzodiazepine (clobazam) or to reduce the doses of clonazepam.
77. LONG-TERM STEROID TREATMENT IN TWO PATIENTS WITH ACQUIRED EPILEPTIC APHASIA Roberto H. Caraballo, Eugenio Grillo, Ana M. Soprano, and Natalio Fejerman, Buenos Aires, Argentina Among a larger series of cases with acquired epileptic aphasia (AEA) we selected for treatment two patients on the basis of having been nonresponsive to different antiepileptic drugs and remaining aphasic for a long time. The age of onset of disease was 6 years in both cases and the delay between onset of AEA and steroid treatment was 15 and 18 months. At this time their sleeping EEGs still demonstrated continuous spike-and-wave activity covering over 85% of the slow sleep recording. Prednisone (1 mg/kg/day) was continued for 6 and 8 months. EEG normalized after 1 month of treatment in the first case. Discharges were less frequent after 2 months, with EEG becoming normal at 6 months in the second. Language response was seen much later: improved comprehension and isolated words appeared after 2 and 3 months, respectively. Almost normal language was recovered at 6 and 8 months of daily steroid treatment. In accordance with the results of other authors, we think that high-dose, prolonged steroid treatment should be attempted
364 PEDIATRIC NEUROLOGY Vol. 8 No. 5
in patients with AEA nonresponsive to antiepileplk drugs, iw cluding wdproic acid, cthosuximide, and benzodia/cpinc~. The first sign to look for is improvement of EEG and ~ c ,hould ,a air for it at least 2-3 months befl~re considering it to bc a m:atmenl failure. Spontaneous remission or changes in EEG abnormalities or in language are probably not operating in thesc 2 cases, considering the long period of persistent clinico-electroencephatographic features of AEA elapsed unlil steroid treatment was started.
78. CONGENITAL CEREBELLAR HYPOPLASIA WITH GRANULAR CELL ATROPHY I. Pascual-Castroviejo, M. Gutierrez, C. Morales Bastos, 1. Gonzalez Mediero, A. Martinez Bermejo, and J. Arcas. Madrid, Spain Eleven children (6 males, 5 females) are presented with cerebellar hypoplasia or atrophy and primary degeneration of the granular cell layer. This disease is a hereditary condition of a recessive autosomal nature and has definite clinical features: hypotonia, strabismus, very slow motor development, nonprogressive ataxia, retarded language development with dysarthria, and mental retardation. All neuroradiologic studies, pneumoencephalography, CT, and MRI, disclosed severe cerebellar hypoplasia. MRIs are the most revealing test and present great similarity among the cases. Necropsy study was performed in 3 of I I patients, and cerebellar hypoplasia with cell loss of the granular cell and diverse abnormalities of the Purkinje cells, was found in 2 patients. The third patient with a histologic study was a gM, sister of one of the 2 patients described above, who had a similar but shorter clinical course, dying at 3 months of age; the necropsy study demonstrated only localized atrophies of the cerebellar folia without microscopic changes in the granular cell layer. Her sister lived to age 5 years and presented with severe cerebellar hypoplasia with typical changes in the granular cell layer and Purkinje cells. The findings observed in our series and the study of cases described in the literature, suggest to us that there are two forms or types of this disease that differ mainly in that one form presents with microcephaly and lower mental level and the other evolves with a normal cephalic size and less severe mental retardation.
79. HYPOMELANOSIS OF ITO: MR][ RESULTS IN 14 PATIENTS A . L Martfnez Jim6nez, 1. Pascual-Castroviejo, E. Mufioz Hiraldo, A. Tendero, C. S~inchez Pina, and M.R. Garcfa Melian, Madrid, Spain During the last 25 years, 68 patients with hypomelanosis of Ito (HI) were studied in our service. MRI was performed in 14 patients (7 males, 7 females). MRI anomalies were found in 8 patients (57%). Intracerebral changes are located in any zone of the brain (i.e., cerebral hemispheres, basal ganglia, brainstem, and less frequently in the cerebellum). These alterations demonstrate several types of lesions, cortico-subcortical changes of the tuberous type (similar to those observed in tuberous sclerosis), hemimegalencephaly, changes of the type of cerebral heterotopies located in white matter of any zone of the cerebral hemispheres, periventricular white matter changes, retarded myelination, and cortico-subcortical atrophy of the temporal lobes. Although most lesions are observed on Tl-weighted images, the